Long QT syndrome: Cellular basis and arrhythmia mechanism in LQT2

Craig T. January, Qiuming Gong, Zhengfeng Zhou

    Research output: Contribution to journalReview article

    96 Scopus citations

    Abstract

    HERG Channel Dysfunction in LQT2. LQT2 is one form of the congenital long QT syndrome. It results from mutations in the human ether-a-go-go-related gene (HERG), and more than 80 mutations, usually causing single amino acid substitutions in the HERG protein, are known. HERG encodes the ion channel pore-forming subunit protein for the rapidly activating delayed rectifier K+ channel (IKr) in the heart. This review summarizes current findings about mutations causing LQT2, the mechanisms by which mutations may cause the clinical phenotype of a reduction in IKr and a prolonged QT interval, and how this may be involved in the generation of ventricular arrhythmias.

    Original languageEnglish (US)
    Pages (from-to)1413-1418
    Number of pages6
    JournalJournal of cardiovascular electrophysiology
    Volume11
    Issue number12
    DOIs
    StatePublished - Jan 1 2000

    Keywords

    • Arrhythmia
    • Cardiac action potentials
    • HERG
    • Long QT syndrome
    • Potassium channels
    • Sudden death

    ASJC Scopus subject areas

    • Cardiology and Cardiovascular Medicine
    • Physiology (medical)

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